Growth pattern and growth prediction of body height in children with mucopolysaccharidosis type II.

AIM: Our goal was to evaluate the level, degree and direction of deviation in the ontogenesis of patients with mucopolysaccharidosis type II (MPS II) in comparison with the healthy population. MATERIAL AND METHODS: The anthropometric data of a longitudinal study on 28 patients with MPS II, aged from 0.5 to 21 years, were used to analyse the general growth patterns in terms of height, weight and head circumference. The growth trend was assessed with the straight-line regression model. The mathematical structural growth model was used to evaluate the structure of body height growth. RESULTS: A statistically significant negative growth trend for all features was found. Analysis of development structure revealed an earlier onset of the adolescent growth spurt among healthy boys and a lower current velocity of growth than expected values. CONCLUSION: During the first 3 years of life, all observed anthropometric features grew faster than normal. They slowed down by the end of the third year and, in subsequent years, reached lower values when compared with the reference charts. The values obtained from the BTT model showed the structure of body height growth, with particular emphasis on the pubertal spurt, was significantly different from the reference charts.

The height-, weight-, and BMI-for-age of Polish school-aged children and adolescents relative to international and local growth references.

BACKGROUND: The growth of children is an indicator of health and society's wellbeing. Growth references are useful in monitoring a child's growth, which is a very important part of child care. Poland's growth references are not updated regularly. Although several growth reference ranges have been developed in Poland over recent years, sampling was restricted to urban populations of major cities. The aim of this study was to assess how well Polish children match with, or diverge from, regional charts and to compare them with international growth references. METHODS: Four Polish and two international (WHO 2007 and USCDC2000) growth references were used to calculate the height, weight and BMI z-scores in a recent, large, population-representative sample of school-aged children and adolescents in Poland. The distributions of z-scores were analysed with descriptive and inferential statistical methods. RESULTS: Mean height z-scores calculated with the use of the WHO 2007 and USCDC2000 references were positive and significantly different from zero over the entire age range. The mean height z-score was closest to zero in the Poznan reference for boys (0.05) and Warszawa reference for girls (0.01). Median weight z-scores were positive under all weight references over the entire age range with only the exception of 18-year-old girls' weight z-score calculated relative to USCDC2000. Median BMI z-scores were positive in males in early childhood, decreasing with age. In the case of girls, the median BMI z-score calculated using WHO 2007 and USCDC2000 was close to zero in early childhood, decreased in adolescents and reached minimum values at age 18 years. Median BMI z-scores calculated with the use of the Lodz reference fluctuated between 0.05 and 0.2 over the studied age range. CONCLUSIONS: In this contemporary sample of Polish school-aged children, distributions of height, weight and BMI differed from those of children from the international growth references. These differences should be considered when using the references. There exist certain limitations to the analysis of height, weight, and BMI z-scores when Polish regional references are used.

[Assessment of the physical development in children with short bowel syndrome based on selected anthropometric parameters]. / Ocena rozwoju fizycznego dzieci z zespolem krótkiego jelita na podstawie wybranych parametrow antropometrycznych.

INTRODUCTION: Short bowel syndrome (SBS) results from partial surgical resection of small bowel and requires parenteral nutrition. Aim of the study was to assess physical development and nutrition status of children with SBS. MATERIAL AND METHODS: The study sample included 176 patients (56 girls and 120 boys) aged from 3 to 10 years. All measured anthropometric parameters were standardized for gender using the Polish body height, weight, head and chest circumference reference charts. All measurements were taken in the Division of Anthropology in Children's Memorial Health Institute according to the standard technique. z-scores were calculated. RESULTS: The statistically significant differences between children with SBS and healthy population in anthropometric features were found. CONCLUSIONS: Physical development of children with SBS is slower than in their healthy peers. The differences were particularly evident among boys. Periodic anthropologic assessment is useful in adjusting energy intake to the individual needs.

Anthropometric data of 14 patients with mucopolysaccharidosis I: retrospective analysis and efficacy of recombinant human alpha-L-iduronidase (laronidase).

OBJECTIVES: Our goal was to evaluate growth patterns in terms of body height, weight, head and chest circumference in patients with mucopolysaccharidosis type I (MPS I) without treatment and after enzyme replacement therapy (ERT) with alpha-l-iduronidase (laronidase). PATIENTS AND METHODS: Anthropometric features of 14 patients with MPS I were followed from birth until the introduction of ERT (group 1-1st year of life, group 2 3rd year of life), after 52-260 weeks of ERT and periodically during treatment. The data since birth until beginning of treatment was obtained by retrospective review of patients' charts. Patients received intravenous laronidase at 100 U/kg (0.58 mg/kg) weekly for 52-260 weeks. RESULTS: Patients from group 1 (n=7) and group 2 (n=7) had similar characteristics at the time of birth but showed significant difference when compared with healthy population. Growth patterns were associated significantly with the MPS I at birth. After 96-260 weeks of ERT, patients receiving laronidase (group 1) compared with group 2 did not show statistically significant improvement. CONCLUSIONS: Anthropometric features of patients with MPS I significantly differ from the healthy population. Children with MPS I grew considerably slower, and differences between healthy and affected children increased with age. In studied patients with MPS I, laronidase did not appear to alter the growth patterns.

[Differences in the head and body structure among children with the ataxia-telangiectasia syndrome]. / Róznice w budowie glowy i ciala dzieci z zespolem ataksja-teleangiektazja.

INTRODUCTION: The ataxia-telangiectasia syndrome (AT, Louis-Bar syndrome) is one of the primary immunodeficiency disorders with genetically determined spontaneous chromosome instability. AT coexist with progressive cerebellar ataxia, sinopulmonary infections, skin disorders, including telangiectasia, radiosensitivity and tendency to recurrent respiratory infections and increased cancer risk. The aim of the study was the finding of the differences in the structure of the head and body between the group of males and the group of females, and to find differences between the group of children with AT and their healthy peers. MATERIAL AND METHODS: The study sample consists of 46 children with AT, including 20 girls and 26 boys. Measurements of 15 head features and 13 morphological features were taken with the current technique of anthropological measurement. The material was described in the form of arithmetic means and standard deviation score after the standardization and was compared in sex groups. In cases where there were no significantly statistical differences, the data were combined and compared with the biological system of reference which was the Dziecko poznanskie '90. RESULTS: In all of the characteristics, both head and body features statistically significant differences between subjects with AT and standards were found. There were no statistically significant differences in most features (with the exception of five) among a group of males and females. Therefore, to the further analysis the two groups were combined. CONCLUSIONS: Patients with Louis Bar syndrome were significantly smaller and slimmer than their peers. In five characteristics such as arm circumference, length of the upper limbs, nose height, lip length and lip height statistically significant differences between male and female group were found. Lip height was lower in a group of females. Other characteristics in a male group were lower than these in female one.